High levels of urinary porphyrins in the urine are due to porphyria. Porphyria is a disorder of porphyrin metabolism characterized by increased excretion of porphyrins and porphyrin precursors in the urine and feces. Porphyrias are congenital disorders caused primarily by deficiencies in various enzymes related to heme synthesis and have a family history of occurrence. What are the tests for protoporphyria? 1. Hematology examination Hemoglobin and erythrocyte volume may be slightly lower than normal. MCV is lower than normal or normal, and MCH is mildly reduced. The life time of red blood cells is normal. 2.Bone marrow examination The degree of bone marrow proliferation and bone marrow cell hematopoiesis are within the normal range. 3.Red blood cell examination The free protoporphyrin in red blood cells exceeds the normal range mostly in 5.4 to 81.0μmol/L is the main diagnostic basis of this disease. Protoporphyrin in plasma is increased, and protoporphyrin in stool is increased or normal. Red fluorescence can be seen on examination of red blood cells in peripheral blood or bone marrow with a fluorescence microscope. This method is easy to use and reliable. 4. Other tests Iron kinetic tests show that the conversion rate of plasma iron and iron utilization are normal. Urine is normal in color and does not contain protoporphyrin. Free protoporphyrin (FEP) in red blood cells is significantly increased, mostly in the range of 5.4 to 81.0μmol/L (normal <0.9), while FEP due to iron deficiency anemia rarely exceeds 5.4μmol/L. In carriers of genetic defects without clinical symptoms, FEP is in the range of 0 to 3.6μmol/L; in patients with severe protoporphyrin liver disease, FEP generally exceeds 24μmol/L. Peripheral blood is visible under fluorescence microscopy with red fluorescence from erythrocytes, accounting for 5% to 25%, positive blood protoporphyrin, normal urine color without porphyrins, and significantly increased fecal protoporphyrin. Ultrasound, electrocardiogram, and X-ray can be done optionally according to the clinical manifestation symptoms and signs.